Recurrent or periodic fever is seen frequently in pediatric medicine. Diagnostic criteria of PFAPA syndrome is characterized by fever, cervical lymphadenopathy, aphthous stomatitis and pharyngitis. First case was a 8 year-old girl and she had recurrent attacks of fever, pharyngitis and aphthous stomatitis in every 3-4 weeks. Fever lasted 5-6 days and no response to antibiotics was received. Acute phase reactants elevated during the attacks and returned to normal between attacks. Symptoms of the patient dramatically returned to normal after single dose of prednisone. Second case was 3.5 year-old boy and he had recurrent attacks of membranous tonsillitis and high fever once a month. Cervical lymphadenopathy was observed in some of the attacks but without any aphthous stomatitis. Fever lasted 5-6 days despite antibiotic use and acute phase reactants elevated. Symptoms of the patient returned to normal after a single dose of prednisone. We want both to emphasize PFAPA syndrome with two atypic patients and also review the literature.